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Pleomorphic rhabdomyosarcoma arising in association with the right pulmonary artery.

Pulmonary artery rhabdomyosarcomas are rare lesions, with a small number of cases reported in the literature. We report a case of pulmonary artery rhabdomyosarcoma that arose in the right pulmonary artery. The patient presented with cough, chest pain, and hemoptysis and was found to have a sarcoma arising in the right pulmonary artery and extending distally within the pulmonary vasculature into the right lung. The gross pathologic features, microscopic appearance, immunohistochemical profile, and computed tomographic and electron microscopic findings are presented. The site of origin and differential diagnosis of these lesions are discussed.

Adult↗

Testicular embryonal rhabdomyosarcoma diagnosed by fine needle aspiration cytology. A report of two cases.

During a 12-year period, 1980-1992, two cases of testicular embryonal rhabdomyosarcoma were diagnosed by fine needle aspiration cytology at the Shiraz University Hospitals. The smears revealed various types of cells including spindle cells, strap cells, rhabdomyoblasts, round cells and tadpole cells with a high nuclear/cytoplasmic ratio. Cross-striations were noticed in the cytoplasm at higher magnification under oil immersion. The cytologic diagnoses of embryonal rhabdomyosarcomas were confirmed on histologic sections.

Biopsy, Needle↗

Embryonal rhabdomyosarcoma of the orbit in a 35-year-old man.

A 35-year-old man had rapidly progressive proptosis of the right eye with associated chemosis over a period of several weeks. Computed tomography demonstrated a solid extraconal mass in the inferior anterior right orbit. Pathologic examination revealed the lesion to be an embryonal rhabdomyosarcoma. Consistent with the diagnosis, immunohistochemical assays demonstrated positive staining with myoglobin, desmin, and muscle-specific actin. The lesion grew rapidly and was further surgically excised. Subsequently, treatment with radiation and chemotherapy was initiated. Primary embryonal rhabdomyosarcoma of the orbit is an extremely rare tumor in adults, and, to our knowledge, this patient represents the oldest individual reported to have developed such a tumor, as documented by immunohistochemical analysis.

Adult↗

Rhabdomyosarcoma in children: clinical analysis of 20 cases.

Rhabdomyosarcoma is the most common soft tissue sarcoma in children. Twenty patients who were treated for rhabdomyosarcoma at this institution from 1979 to 1989 were reviewed. All of the patients were under 14 years of age. Two patients had tumors in the head and neck region (excluding the orbit), four in the orbit, seven in the trunk and extremities, five in the intra-abdominal or retroperitoneal region, and two in the urinary bladder. Eleven tumors were subclassified as the embryonal type, five as the alveolar type, one as the pleomorphic type, and the other three were not subclassified. Most patients received combined treatment with surgery and chemotherapy and/or irradiation. The overall one-year survival rate was 54% and the two-year survival rate was 31%. Several prognostic factors were identified. Longer-term survivors were younger than six years, had a histology of the embryonal subtype, were clinical group I or had tumors located in the orbit. Aggressive treatment is necessary to achieve a better outcome.

Adolescent↗

[A case of embryonal rhabdomyosarcoma of the cheek in adolescence. Its cytological diagnosis by fine-needle aspiration via the gingival fornix].

The authors present a case of embryonal rhabdomyosarcoma of the cheek in a young patient. The histological diagnosis, on the tumoral mass, was preceded by cytological researches with fine needle aspiration biopsy, carried out on the gingival fornix. The cytology, by immunocytochemical techniques, made possible the diagnosis of rhabdomyosarcoma. Therefore the authors stress the high capacity of these cytochemical and immunocytochemical methods to demonstrate, not in a invasive way, the small round cell tumors of the skull and the neck in the youngs. This early diagnosis may orient the clinical and therapeutic management of the case.

Adolescent↗

[Embryonal rhabdomyosarcoma of the orbit].

The paper shows the case of a 19-years-old patient, who presented two month before the observation took place marked exophthalmos at the left eye with general state alteration. Computerised tomography pointed out a tumoral formation having 4 centimeters in diameter, which pushed off the anterior ocular globe, eroding to the left frontal lobe. The anatomopathological exam evidenced the embryonal rhabdomyosarcoma. So, every unilateral exophthalmos of the child or teenager must lead the diagnosis of embryonal rhabdomyosarcoma. The therapy consists of exeresis biopsy, followed by complex radiotherapy and chemotherapy. The orbital exenteration, a multilant intervention, does not guarantee for the further evolution of the affection.

Adult↗

Ongoing progress in the treatment of childhood rhabdomyosarcoma.

During the past 2 decades, several different multidisciplinary treatment studies of childhood rhabdomyosarcoma have been conducted, some as single-institution protocols and others as multi-institution cooperative endeavors. Much of our current understanding of the natural history and histopathology, as well as response to treatment, is derived from these studies. This understanding has allowed for the continued evolution of treatment, which has already resulted in a dramatic increase in overall survival rates. Current treatment trials are aimed at maintaining high cure rates while reducing treatment-related morbidity, and exploring innovative regimens to improve the outlook of poor-risk patients. The improved outcome in children with rhabdomyosarcoma is a tribute to the multi-institutional, multidisciplinary approach to cancer research and treatment.

Antineoplastic Combined Chemotherapy Protocols↗

[Cardiac rhabdomyosarcoma. Its echographic diagnosis].

We report a case of cardiac rhabdomyosarcoma whose initial clinical features were fever and palpitations due to documented ventricular tachycardia. Sequential two-dimensional echocardiographic studies pointed out the presumed diagnosis, showing intracavitary masses at multiple sites appearing within a short period of time. The postmortem examination confirmed the diagnosis of rhabdomyosarcoma.

Adult↗

[A infant case of rhabdomyosarcoma arising from the left iliac region with complete remission for 2 years after combination therapy].

A case of rhabdomyosarcoma in a 14-year-female is reported herein. The patient developed left inguinal pain and tumor. She was initially seen by her local medical doctor, who biopsied her left inguinal tumor with a pathological report of malignancy. She was referred to the Ryukyu University Hospital in July, 1990. CT and MRI demonstrated a large tumor 10 cm in diameter arising from the left iliac region. The tumor was extirpated and the pathological report indicated rhabdomyosarcoma (alveolar type). The patient was treated with VAC chemotherapy and local irradiation. Her course has been uneventful for the past 2 years without any clinical manifestation of recurrence since surgery.

Adolescent↗

Cholinesterase response in the rhabdomyosarcoma tumor and small intestine of the BALB/c mice and the radioprotective actions of exogenous ATP after lethal dose of neutron radiation.

The rhabdomyosarcoma tumors were subjected to different doses of 2.0, 3.8 and 7.0 Gy from a neutron beam facility p(66 MeV)/Be. Elevated levels of cholinesterase activity are observed in which there is a correlation between the different doses of neutron radiation and the augmentation response of this enzyme. The increase of cholinesterase activity after 7 Gy neutron irradiation as a feature of involvement in the homeostatic mechanism maintaining the proper choline/acetylcholine ratio in the cell is also observed at 1 and 24 h in both tissues, rhabdomyosarcoma and small intestine. The activity of the enzyme after neutron irradiation with prior administration of ATP showed smaller increases when compared with increases observed after neutron irradiation alone. Moreover in the present work the protective mechanism of ATP in the response of cholinesterase activity is marked differential between both, normal and tumoral tissue and correlated inversely with the administered of the following concentrations of exogenous ATP (8, 25, 80, 250, and 700 mg/kg body weight) prior to exposure to 7 Gy neutron radiation. These results reflect the radioprotective ability of exogenous ATP to exert a number of metabolic adaptations as a defense mechanism in which the cell exposed to neutron radiation could remain viable because the injury is potentially repairable.

Adenosine Triphosphate↗

Primary pulmonary rhabdomyosarcomas: a clinicopathologic and immunohistochemical study of three cases.

Three cases of primary pulmonary rhabdomyosarcoma in adults are presented. The patients were all men between the ages of 57 and 78 yr (mean 67.5). All patients presented with symptoms referable to their tumors, including cough, shortness of breath, pleuritic chest pain, and weight loss. In one patient, a history of tobacco and alcohol abuse was obtained. Anatomically, two tumors were located in the left upper lobe and one in the left lower lobe. Grossly, the tumors ranged in size from 6 to 11.5 cm and were tan-gray, firm masses with areas of necrosis and hemorrhage. Histologically, the tumors were characterized by a spindle cell proliferation admixed with areas showing a pleomorphic cell population with numerous rhabdomyoblasts and areas of hemorrhage and necrosis. Immunohistochemically, all three tumors showed strong positivity with desmin and myoglobin antibodies and negative staining with antibodies against keratin, epithelial membrane antigen, and S-100 protein. All patients had a fatal outcome. Two patients died a few days after admission with respiratory distress; the third one died 2 years after diagnosis with widely metastatic disease. Autopsy findings in all cases disclosed disseminated metastases to multiple abdominal and thoracic organs. Primary pulmonary rhabdomyosarcoma should be considered in the differential diagnosis of poorly differentiated pulmonary neoplasms in adults and should be distinguished from other primary and metastatic sarcomas.

Aged↗

Cultured pig rhabdomyosarcoma cells with a deletion of the Xq24-qter chromosome region: an immunochemical and cytogenetic characterization.

A pig rhabdomyosarcoma cell line (PRUM59) was established, and the immuno(histo)chemical and cytogenetic characterization of these cells was determined. At various swine farms in the Netherlands, pigs were observed that had solitary or multiple skin nodules, which were diagnosed as rhabdomyosarcomas. Cells of a tumor derived from a 3.5-week-old female pig were cultured for immunochemical and cytogenetic analyses. The cell line had characteristic features of undifferentiated muscle cells, similar to those observed in tumor tissue sections; they contained titin, a high-molecular weight protein specific for striated muscle, as dot-like aggregates and as filaments, desmin filaments and cross-striations, smooth muscle actin stress fibers, and vimentin filaments. The cells stained positively for striated muscle actin and tropomyosin as well. The immunohistochemical staining results were supported by results of immunoblotting experiments. Karyotyping of the cells revealed a deletion of a major part of Xq24-qter, a part of the long arm of 1 of the 2 X chromosomes. The other X chromosome and all autosomes appeared to be normal.

Animals↗

[Rhabdomyosarcoma of the bladder: a case report].

A 15-year-old man with the chief complaint of general fatigue was referred to our hospital on November 11, 1993. Bilateral percutaneous nephrostomy was performed for postrenal anuria. X-ray examinations revealed a huge intrapelvic tumor, and it was histopathologically diagnosed as rhabdomyosarcoma by transrectal needle biopsy. He was initially treated with combination chemotherapy regimen of vincristine, actinomycin-D and cyclophosphamide (VAC therapy). Pelvic exenteration was performed on December 15, 1993. Histopathological findings were alveolar rhabdomyosarcoma with degenerative change and partial necrosis. After the operation, he was given two course of VAC therapy. In May, 1994, brain metastasis occurred, so 4 courses of VAC therapy were administered. For a very short period, neurological symptoms improved, but he died of pneumonia on November 15, 1994.

Adolescent↗

Embryonal rhabdomyosarcoma of the uterine corpus mistaken for small cell carcinoma: a case report.

Pure rhabdomyosarcomas of the female genital tract mostly occur in infancy and childhood, in the form of sarcoma botryoides (a variant of embryonal rhabdomyosarcoma), with vagina and cervix as typically involved sites. Such tumors rarely occur in the uterine corpus and cervix of adults. We would like to report a pure embryonal rhab-domyosarcoma of the uterine corpus that arose in a 31-year-old, gravida 1, para 1, female patient with widespread bony metastasis, and which was originally mistaken for metastatic small cell carcinoma to bone marrow.

Adult↗

Reconstructive options in genitourinary rhabdomyosarcoma.

PURPOSE: We attempted to develop a rational and consistent scheme for surgical reconstruction in patients with genitourinary rhabdomyosarcoma. MATERIALS AND METHODS: We reviewed the records of 35 patients with resectable genitourinary rhabdomyosarcoma treated from 1970 to 1993. RESULTS: Primary sites included bladder in 11 cases, prostate in 13, vagina/uterus in 9 and pelvic tumors of uncertain origin in 2. A total of 33 patients underwent surgery, including partial and radical cystectomy in 17 (bowel conduit diversion in 10, continent urinary diversion in 6 and ureterosigmoidostomy in 1). Overall 30 of the 33 surgical patients are free of disease 4 months to 24 years after diagnosis. CONCLUSIONS: A nonrefluxing colon conduit is appropriate at cystectomy. Continent diversion fashioned from the original conduit may be planned as the patient achieves a durable disease-free status.

Adolescent↗

[A case of adult chest wall rhabdomyosarcoma].

A 68-year-old woman was admitted to our hospital with a left anterior chest wall tumor. A percutaneous needle biopsy was performed, leading to a pathological diagnosis of pleomorphic rhabdomyosarcoma. A wide en bloc excision of the left anterior chest wall was performed, including the left sixth and seventh ribs. The defect of the chest wall was closed with Marlex mesh. Postoperative adjuvant chemotherapy (CYVADIC) was administered. The patient remained free of local recurrence two years after surgery. We conclude that wide en block excision and close follow-up are important in the management of rhabdomyosarcoma of the chest wall because of a high risk of recurrence.

Aged↗

Alpha 2a-interferon-induced differentiation of human alveolar rhabdomyosarcoma cells: correlation with down-regulation of the insulin-like growth factor type I receptor.

Rhabdomyosarcoma, a tumor of skeletal muscle origin, appears developmentally arrested at an early stage in the myogenic differentiation pathway. The proliferation of an alveolar rhabdomyosarcoma cell line Rh30 is dependent on the insulin-like growth factor (IGF) II/IGF-I receptor (IGF-IR) signaling pathway and is highly sensitive to recombinant human IFN-alpha 2a, which induces growth arrest and differentiation of these malignant myoblasts. IFN-alpha 2a-induced growth arrest of Rh30 cells was observed within 48 h, and reduction in colony formation was obtained with an IC50 of 0.81 IU/ ml for 72 h exposure. Down-regulated expression of IGF-IR was apparent by 24 h after initiation of IFN-alpha 2a treatment. Furthermore, an initial increase followed by reduced expression of MyoD, in concert with elevated expression of myogenin, increased frequency of skeletal muscle myosin-positive cells, and the formation of multinucleated cells, indicated an enhancement of differentiation of Rh30 cells in the presence of IFN-alpha 2a. To probe the role of IGF-IR in the differentiation of Rh30 cells along the myogenic lineage, the effect of antisense RNA-mediated reduction of endogenous IGF-IR on growth and expression of muscle-specific proteins was determined. Rh30 cells transfected to stably express antisense IGF-IR (clone AS [symbol: see text] 23)showed significant reduction in growth rate, decreased expression of IGF-IR protein, increased expression of MyoD, myosin heavy chain, and an increased number of multinucleated cells in comparison to the parental line. These data are consistent with overexpression of IGF-IR inhibiting differentiation. IFN-alpha 2a treatment of AS [symbol: see text] 23 cells further induced both MyoD and myogenin expression, thereby allowing cells to proceed further downstream of the differentiation pathway.

Cell Cycle↗

Rhabdomyosarcoma of head and neck--an analysis of 24 cases.

Clinical records of 24 cases or rhabdomyosarcoma involving head and neck region were studied. Majority of the patients (83.3%) were under five years of age. The common sites of involvement were the middle ear (50%) and the nasopharynx (17%). Embryonal variety was noted in majority of patients (83.3%). Pain and swelling in the post aural or pre-auricular region was the commonest mode of presentation. Last four cranial nerves were involved in eight patients and four patients had extension of the tumor in the posterior cranial fossa. Twenty patients having embryonal rhabdomyosarcoma received combination of chemotherapy and radiotherapy. Half of these remained tumor free upto six months. Surgical extirpation was done only in two patients, where the disease was localised. The tumor in these two patients was of pleomorphic variety and did not recur till two years after surgery.

Adolescent↗